MST1 functions as a key modulator of neurodegeneration in a mouse model of ALS
DC Field | Value | Language |
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dc.contributor.author | Lee, Jae Keun | - |
dc.contributor.author | Shin, Jin Hee | - |
dc.contributor.author | Hwang, Sang Gil | - |
dc.contributor.author | Gwag, Byoung Joo | - |
dc.contributor.author | McKee, Ann C. | - |
dc.contributor.author | Lee, Junghee | - |
dc.contributor.author | Kowall, Neil W. | - |
dc.contributor.author | Ryu, Hoon | - |
dc.contributor.author | Lim, Dae-Sik | - |
dc.contributor.author | Choi, Eui-Ju | - |
dc.date.accessioned | 2021-09-05T23:40:18Z | - |
dc.date.available | 2021-09-05T23:40:18Z | - |
dc.date.created | 2021-06-14 | - |
dc.date.issued | 2013-07-16 | - |
dc.identifier.issn | 0027-8424 | - |
dc.identifier.uri | https://scholar.korea.ac.kr/handle/2021.sw.korea/102696 | - |
dc.description.abstract | Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder characterized by loss of motor neurons. Dominant mutations in the gene for superoxide dismutase 1 (SOD1) give rise to familial ALS by an unknown mechanism. Here we show that genetic deficiency of mammalian sterile 20-like kinase 1 (MST1) delays disease onset and extends survival in mice expressing the ALS-associated G93A mutant of human SOD1. SOD1(G93A) induces dissociation of MST1 from a redox protein thioredoxin-1 and promotes MST1 activation in spinal cord neurons in a reactive oxygen species-dependent manner. Moreover, MST1 was found to mediate SOD1(G93A)-induced activation of p38 mitogen-activated protein kinase and caspases as well as impairment of autophagy in spinal cord motoneurons of SOD1(G93A) mice. Our findings implicate MST1 as a key determinant of neurodegeneration in ALS. | - |
dc.language | English | - |
dc.language.iso | en | - |
dc.publisher | NATL ACAD SCIENCES | - |
dc.subject | AMYOTROPHIC-LATERAL-SCLEROSIS | - |
dc.subject | INCREASED OXIDATIVE DAMAGE | - |
dc.subject | MOTOR-NEURON DEATH | - |
dc.subject | PROTEIN-KINASE | - |
dc.subject | AUTOPHAGY | - |
dc.subject | ACTIVATION | - |
dc.subject | APOPTOSIS | - |
dc.subject | CELLS | - |
dc.subject | STE20 | - |
dc.subject | MICE | - |
dc.title | MST1 functions as a key modulator of neurodegeneration in a mouse model of ALS | - |
dc.type | Article | - |
dc.contributor.affiliatedAuthor | Lee, Jae Keun | - |
dc.contributor.affiliatedAuthor | Choi, Eui-Ju | - |
dc.identifier.doi | 10.1073/pnas.1300894110 | - |
dc.identifier.scopusid | 2-s2.0-84880389532 | - |
dc.identifier.wosid | 000322086100086 | - |
dc.identifier.bibliographicCitation | PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, v.110, no.29, pp.12066 - 12071 | - |
dc.relation.isPartOf | PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA | - |
dc.citation.title | PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA | - |
dc.citation.volume | 110 | - |
dc.citation.number | 29 | - |
dc.citation.startPage | 12066 | - |
dc.citation.endPage | 12071 | - |
dc.type.rims | ART | - |
dc.type.docType | Article | - |
dc.description.journalClass | 1 | - |
dc.description.journalRegisteredClass | scie | - |
dc.description.journalRegisteredClass | scopus | - |
dc.relation.journalResearchArea | Science & Technology - Other Topics | - |
dc.relation.journalWebOfScienceCategory | Multidisciplinary Sciences | - |
dc.subject.keywordPlus | AMYOTROPHIC-LATERAL-SCLEROSIS | - |
dc.subject.keywordPlus | INCREASED OXIDATIVE DAMAGE | - |
dc.subject.keywordPlus | MOTOR-NEURON DEATH | - |
dc.subject.keywordPlus | PROTEIN-KINASE | - |
dc.subject.keywordPlus | AUTOPHAGY | - |
dc.subject.keywordPlus | ACTIVATION | - |
dc.subject.keywordPlus | APOPTOSIS | - |
dc.subject.keywordPlus | CELLS | - |
dc.subject.keywordPlus | STE20 | - |
dc.subject.keywordPlus | MICE | - |
dc.subject.keywordAuthor | neurotoxicity | - |
dc.subject.keywordAuthor | ROS | - |
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